Developing a Sickle Africa Data Coordinating Center (SADaCC)

项目概要/摘要（30行） 据估计，每年有305，800名婴儿出生时患有镰状细胞病（SCD） 全球近75%的新生儿出生在撒哈拉以南非洲（SSA）。尽管如此 由于发病率高，目前在任何撒哈拉以南非洲国家都没有有效的公共卫生方案， 专注于SCD。因此，在SSA中，高达90%的SCD婴儿被认为 五年后死去。来自西方的证据表明， 因为新生儿筛查和青霉素预防可以减少约70%的 SCD儿童的死亡率。然而，成人SCD患者的死亡率在 由于额外的衰弱性并发症，US没有改善。因此，有一个主要的 需要进行研究，以帮助开发有效的治疗方法，在整个生命周期的SCD的所有部分 of the world.纵向队列研究是最科学严谨的方法， 了解风险因素以及健康和疾病结果。高发病率， SSA有望促进流行病学、转化和临床研究 SCD的研究，类似于心脏的Frachial研究。 此U24应用程序正在寻求建立一个数据协调中心（DCC）， 镰刀非洲数据协调中心（SADaCC），以支持 非洲的SCD网络。这一总体目标将通过以下四个方面来实现： 具体目标：目标1：提供全面协调和行政支助， 将包括开发和维护操作程序;创建和 SCD本体管理。目标2：建立和确保维持一个 一个集中的电子镰状血红蛋白病数据库， 包括适当的同意程序和提供数据 管理和生物信息学技能。目标3：提供生物统计学研究 发展、分析和医疗专业知识。目标4：提供2年的发展 一个为期一年的试点/规划阶段和一个为期一年的实施阶段。 我们相信，我们的团队的a）以前的经验，作为一个协调中心， H3非洲联合会，B）大学杰出的体制环境， 开普敦（UCT），c）UCT临床研究中心在 研究/数据管理和H3 ABioNet在生物信息学能力建设方面的作用，以及d） 我们在基础，转化和临床研究方面的记录，以及在SCD方面的培训， SSA，预示着为SCD财团建立最有效的DCC。

Project Summary/Abstract (30 lines) It is estimated that 305,800 babies are born each year with Sickle Cell Disease (SCD) worldwide with nearly 75% of the births occurring in sub-Saharan Africa (SSA). Despite this high incidence, there are currently no effective public health programs in any SSA country focused on SCD. As a consequence, up to 90% of infants with SCD in SSA are believed to die by five years. Evidences from the West indicate that the institution of interventions such as newborn screening and penicillin prophylaxis are credited with the ~70% reduction in mortality rate among children with SCD. However, the death rate in adult SCD patients in the US has not improved, due to additional debilitating complications. There is therefore a major need for research to help develop effective therapies across the life span for SCD in all parts of the world. Longitudinal cohort studies are the most scientifically rigorous methods in understanding of, risk factors, and health and disease outcomes. High disease prevalence in SSA would be expected to facilitate epidemiologic, translational, and clinical research studies on SCD, similar to Framingham Study for the heart. This U24 application is seeking to establish a Data Coordinating Center (DCC) to be called the Sickle Africa Data Coordinating Center (SADaCC), to support the activities of a companion SCD Network in Africa. This overall objective will be accomplished by four specific aims: Aim 1: To provide overall coordination and administrative support that will include development and maintenance of operating procedures; creation and management of SCD Ontology. Aim 2: To create and ensure maintenance of a centralized, electronic sickle hemoglobinopathy database that will include development of protocols, including appropriate consenting procedures and provision of data management and bioinformatics skills. Aim 3: to provide biostatistical, study development, analytic and medical expertise. Aim 4: to provide 2 year development phase, and 1 year pilot/planning phase and a 1 year implementation phase. We believe that our team’s a) prior experience, as a coordinating center, of the activities of the H3Africa Consortium, b) the outstanding institutional environment at the University of Cape Town (UCT), c) the long experience of the UCT Clinical Research Centre in research/data management and H3ABioNet in bioinformatics capacity development, and d) our track record in basic, translational and clinical research, and training focused on SCD in SSA, augurs well to establish the most effective DCC for the SCD consortium.